Cognitive deficits after recovery from thrombotic thrombocytopenic purpura.

BACKGROUND: Patients with apparent complete recovery from thrombotic thrombocytopenic purpura (TTP) often complain of problems with memory, concentration, and fatigue. STUDY DESIGN AND METHODS: Twenty-four patients who were enrolled in the Oklahoma TTP-HUS Registry for their initial episode of TTP, 1995-2006, and who had ADAMTS13 activity of less than 10 percent were evaluated for a broad range of cognitive functions 0.1 to 10.6 years (median, 4.0 years) after their most recent episode. At the time of their evaluation, they had normal physical and Mini-Mental State Examinations and no evidence of TTP. RESULTS: The patients, as a group, performed significantly worse on 4 of the 11 cognitive domains tested than standardized US data from neurologically normal individuals adjusted for age, sex, and education (p < 0.05). These four domains measured complex attention and concentration skills, information processing speed, rapid language generation, and rote memorization. Twenty-one (88%) patients performed below expectations on at least 1 of the 11 domains. No clear patterns were observed between cognitive test results and patients' characteristics or features of the preceding TTP, including age, occurrence of severe neurologic abnormalities, multiple episodes, and interval from an acute episode. CONCLUSION: Patients who have recovered from TTP may have persistent cognitive abnormalities. The abnormalities observed in these patients are characteristic of disorders associated with diffuse subcortical microvascular disease. Studies of larger patient groups will be required to confirm these preliminary observations and to determine patient characteristics that may contribute to persistent cognitive abnormalities.

Long-term deficits in health-related quality of life after recovery from thrombotic thrombocytopenic purpura.

BACKGROUND: After recovery from thrombotic thrombocytopenic purpura (TTP), patients commonly describe persistent problems with memory, concentration, and endurance. STUDY DESIGN AND METHODS: The Oklahoma TTP-HUS Registry, an inception cohort of 364 patients, January 1, 1989, through June 30, 2007, has annually evaluated health-related quality-of-life since 1998 with the Short Form (SF)-36, a widely used questionnaire that assesses eight domains of physical and mental health. RESULTS: A total of 128 patients were eligible for this study (age >or= 18 years, alive in January 1998 without prior relapse, survival and no relapse >or=1 year after recovery, no overt disability or additional disorders); 118 (92%) completed the SF-36. At their initial assessment, these patients had significantly worse functioning and well-being than the US population for all domains (p < 0.05). Forty-nine patients who had their initial assessment more than 2 years after recovery had better scores for three domains than 69 patients who had their initial assessment 2 years or less after recovery (p < 0.05). Among these 69 patients, there was no difference between subgroups: 1) idiopathic versus other categories of TTP, 2) the presence or absence of severe ADAMTS13 deficiency, 3) the presence or absence of severe neurologic abnormalities during the episode of TTP, and 4) less than 10 or 10 or more plasma exchange treatments required to achieve remission (p > 0.05). Analysis of serial assessments in 72 (61%) of the 118 patients who had two or more assessments within 5 years of recovery and no intervening relapses demonstrated no significant improvement in any of the domains. CONCLUSION: After recovery from TTP, patients may have persistent cognitive and physical difficulties.